Abstract
Planar cell polarity (PCP) is the uniform orientation and alignment of a group of cells orthogonal to the apical-basal axis within a tissue. Originally described in insects, it is now known that PCP is required for many processes in vertebrates, including directional cell movement, polarized cell division, ciliary orientation, neural tube closure, heart development and lung branching. In this review, we outline the evidence implicating PCP in kidney development and disease focusing initially on the function of PCP in ureteric bud branching and elongation. We then describe how defects in PCP may lead to polycystic kidney disease and discuss a newly identified role for PCP in the kidney filtration barrier.